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Cystic Fibrosis
' Cystic Fibrosis ' Cystic fibrosis is an inherited disease that causes thick, sticky mucus to build up in the lungs and digestive tract. It is one of the most common chronic lung diseases in children and young adults, and may result in early death. The name cystic fibrosis refers to the characteristic scarring (fibrosis) and cyst formation within the pancreas, first recognized in the 1930s. Symptoms Because there are more than 1,000 mutations of the CF gene, symptoms differ from person to person. Symptoms in newborns may include: *Delayed growth *Failure to gain weight normally during childhood *No bowel movements in first 24 to 48 hours of life *Salty-tasting skin Symptoms related to bowel function may include: *Belly pain from severe constipation *Increased gas, bloating, or a belly that appears swollen (distended) *Nausea and loss of appetite *Stools that are pale or clay colored, foul smelling, have mucus, or that float *Weight loss Symptoms related to the lungs and sinuses may include: *Coughing or increased mucus in the sinuses or lungs *Fatigue *Nasal congestion caused by nasal polyps *Recurrent episodes of pneumonia. Symptoms in someone with cystic fibrosis include: **Fever **Increased coughing **Increased shortness of breath **Loss of appetite **More sputum *Sinus pain or pressure caused by infection or polyps Causes Cystic fibrosis (CF) is caused by a defective gene which causes the body to produce abnormally thick and sticky fluid, called mucus. This mucus builds up in the breathing passages of the lungs and in the pancreas, the organ that helps to break down and absorb food. This collection of sticky mucus results in life-threatening lung infections and serious digestion problems. The disease may also affect the sweat glands and a man's reproductive system. Millions of Americans carry the defective CF gene, but do not have any symptoms. That's because a person with CF must inherit two defective CF genes -- one from each parent. An estimated 1 in 29 Caucasian Americans have the CF gene. The disease is the most common, deadly, inherited disorder affecting Caucasians in the United States. It's more common among those of Northern or Central European descent. Most children with CF are diagnosed by age 2. A small number, however, are not diagnosed until age 18 or older. These patients usually have a milder form of the disease. Tests and Diagnosis A blood test is available to help detect CF. The test looks for variations in a gene known to cause the disease. Other tests use to diagnose CF include: *Immunoreactive trypsinogen (IRT) test is a standard newborn screening test for CF. A high level of IRT suggests possible CF and requires further testing. *Sweat chloride test is the standard diagnostic test for CF. A high salt level in the patient's sweat is a sign of the disease. Other tests that identify problems that can be related to cystic fibrosis include: *Chest x-ray or CT scan *Fecal fat test *Lung function tests *Measurement of pancreatic function *Secretin stimulation test *Trypsin and chymotrypsin in stool *Upper GI and small bowel series Treatment An early diagnosis of CF and a treatment plan can increase survival and the overall quality of life. Main care for at cycstic fibrosis is often taken at specialty clinic. When children reach adulthood, they should transfer to a cystic fibrosis specialty center for adults. Treatment for lung problems includes: *Antibiotics to prevent and treat lung and sinus infections. *Inhaled medicines to help open the airways *(DNAse enzyme replacement therapy) to thin mucus and make it easier to cough up *Various vaccines such as the flu vaccine *Lung transplant (if necessary) *Oxygen therapy Care at home should include: *Avoiding smoke, dust, dirt, fumes, household chemicals, fireplace smoke, and mold or mildew *Drinking fluids. *Often exercising http://presenter.multicastmedia.com/links/CFF/Horizon_of_Hope.htm Cystic Fibrosis Care Centers When the Cystic Fibrosis Foundation was established in 1955, most children did not live to attend elementary school. Today, the predicted median age of survival is more than 37 years, thanks—in large part—to the care provided though the national network of CF Foundation-accredited centers. The network provides expert cystic fibrosis care for people living with the disease. In fact, the National Institutes of Health has cited the CF care center network as a model of effective and efficient health care delivery for a chronic disease. The Foundation funds and accredits about 115 cystic fibrosis care centers nationwide. Most care centers have a pediatric and adult program or clinic. Some centers also include affiliate programs. All programs, however, are staffed by dedicated healthcare professionals who specialize in the treatment of cystic fibrosis.Each center undergoes thorough review by the CF Foundation’s Center Committee before it receives accreditation and funding. This review for accreditation occurs every year. Besides taking care of people with cystic fibrosis, care centers also participate in CF clinical research and work to educate healthcare professionals about CF and the care people living with the disease require. Data from people with CF is collected in the national CF Foundation Patient Registry, which is as a rich resource for your CF health care provider and CF researchers to track the health of people living with CF in the United States. Category:Symptoms/Diagnosis